Double-outlet right atrium is a rare congenital cardiac anomaly previously described in association with atrioventricular canal defect. Straddling tricuspid valve is another uncommon anomaly that is almost always associated with a ventricular septal defect. We report the echocardiographic features of a newborn with double-outlet right atrium associated with pulmonary atresia, intact ventricular septum, and right coronary artery ostial atresia. Alternatively, the anatomy can be interpreted as straddling tricuspid valve with intact ventricular septum. The echocardiographic findings were confirmed by cardiac catheterization.
Double-outlet right atrium (DORA) is a rare congenital cardiac anomaly in which the right atrium communicates with both ventricles. This report describes the echocardiographic features of DORA in a newborn with pulmonary atresia and intact ventricular septum associated with atresia of the right coronary artery ostium.
Case Report
A newborn with a prenatal diagnosis of pulmonary atresia was referred to the Harvard Medical School for treatment. A transthoracic echocardiogram demonstrated visceral situs solitus, levocardia, normal segmental cardiovascular anatomy, and pulmonary atresia with intact ventricular septum. The right ventricle was hypoplastic and hypertrophied. The pulmonary arteries were supplied by a tortuous patent ductus arteriosus. The left coronary artery was dilated with to-and-fro flow, and there was no luminal continuity between the right coronary artery and the aortic root ( Figure 1 ; Videos 1 and 2 ). Color Doppler flow mapping demonstrated multiple fistulae between the right ventricle and the coronary arteries.
The tricuspid valve had 2 distinct components separated by redundant mobile tissue attached to the ventricular septal crest with no interventricular communication. The right atrium communicated with the left ventricle through the left-sided tricuspid valve component ( Figures 2 and 3 ; Videos 3 and 4 ) and with the right ventricle through the right-sided tricuspid valve component ( Figure 3 ; Video 4 ), thus DORA. A normal-appearing mitral valve connected the left atrium with the left ventricle. The left-sided tricuspid valve component had chordal attachments to the left ventricular papillary muscles, as typically seen in straddling tricuspid valve. Cardiac catheterization subsequently confirmed the echocardiographic diagnoses and revealed right ventricle-dependent coronary circulation ( Figure 4 ).
The patient underwent a right modified Blalock–Taussig shunt and ligation of the patent ductus arteriosus at age 1 week. The early postoperative course was remarkable for ST-segment depression and hypoxemia, which resolved after pharmacologic augmentation of systemic blood pressure. The remainder of the postoperative course was uncomplicated. An echocardiogram and catheterization at age 11 weeks in anticipation of a bidirectional Glenn shunt demonstrated suprasystemic right ventricular systolic pressure, patent Blalock–Taussig shunt, multiple right ventricle-to-coronary artery fistulae, and good left ventricular function.
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